L-Isoleucine-<wbr/><sup>13</sup>C<sub>6</sub>

L-Isoleucine-13C6

CAT N°: 9004090
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From 117.00 99.45

L-Isoleucine-13C6 is intended for use as an internal standard for the quantification of L-isoleucine by GC- or LC-MS. L-Isoleucine is an essential and branched-chain amino acid.{64732} It is an isomer of L-leucine (Item No. 34342) and a diastereomer of L-alloisoleucine (Item No. 34904) that functions as a nitrogen donor and has roles in glucose consumption, fatty acid metabolism, and immune function.{64732,64733} Urinary levels of L-isoleucine are increased in patients with deficiencies in ?-ketothiolase, short/branched-chain specific acyl-CoA dehydrogenase (SBCAD), or 2-methyl-3-hydroxybutyryl-CoA dehydrogenase (MHBD), inborn errors of metabolism characterized by episodic ketoacidosis and neurological impairments.{64731}

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